Stimulation of 5-aminolevulinic acid metabolism by uroporphyrinogen I in rat liver homogenate

Abstract

The effect of excess uroporphyrinogens I and III, coproporphyrinogen III, and the corresponding porphyrins, on the rate of 4-14C-5-aminolevulinic acid (ALA) metabolism was studied. Experiments were performed with mitochondria-free rat liver homogenates prepared from normal rats. The consumption of labelled 5-aminolevulinic acid was followed by measuring its level in aliquots removed at intervals. The pattern of porphyrinogen synthesis was examined by high pressure liquid chromatography. Only uroporphyrinogen I had an effect; it increased the rate of conversion of ALA and porphobilinogen (PBG) to porphyrinogens. Chromatographic analysis revealed increased synthesis of uroporphyrinogen and heptacarboxylic porphyrinogen. It is believed that this mechanism might explain the lack of ALA and PBG accumulation in erythropoietic porphyria and porphyria cutanea tarda, and the absence of acute porphyria attacks in these conditions.